朗格汉斯组织细胞增多症1例并相关文献复习  

A patient of langerhans cell granulomatosis and review of related literature

作　　者：张荣耀[1] 李娜[1] 张露璐 李章志[1] 宋斌[1] 张霞[1] 万楚成[1] ZHANG Rong-yao;LI Na;ZHANG Lu-lu(Department of Hematology,Taihe Hospital of Shiyan City,Hubei Province,Shiyan 442000,China)

机构地区：[1]湖北省十堰市太和医院血液内科,湖北十堰442000

出　　处：《吉林医学》2020年第4期1017-1019,共3页Jilin Medical Journal

摘　　要：目的:探讨以肺部受累为主要表现的朗格汉斯组织细胞增多症(LCH)的临床表现,影像学特点及诊治要点。方法:回顾性分析2017年确诊为LCH 1例的临床表现、影像学改变、病理特点,总结其病史特点及诊治要点。结果:LCH是一种少见疾病,临床以胸闷、气促为主要表现的多系统损害的肺朗格汉斯组织细胞增多症(PLCH),侵犯骨骼、口腔及黏膜损伤,肺部表现为肺大泡、反复气胸及囊样改变。其诊断主要依靠病理学,治疗手段包括手术、化疗、放射及激素治疗。结论:PLCH早期症状不典型,应及早完善病理学检查,提高诊治效率。Objective This article discussed the clinical manifestations,imaging features,diagnosis and treatment of Langerhans histiocytosis(LCH)with lung involvement as its main manifestation.Method A case of Langerhans histiocytosis diagnosed in the Department of Respiratory Medicine in our hospital in 2017 was retrospectively analyzed in terms of clinical manifestations,imaging changes and pathological features.The characteristics of its history and the main points of diagnosis and treatment were summarized.Results LCH was a rare and stubborn disease with scarcely active treatment till now.The langerhans histicytosis with multiple system damage main clinical manifestations were chest tightness and shortness of breath,of which invades bones,mouth and mucous membranes,the imaging features of lung was pulmonary bllae,repeated pneumothorax and cystic changes.The diagnosis of LCH was based on histological and immunophenotypic examination of lesional tissue.The treatment of LCH included chemotherapy,radiotherapy,and hormone therapy.Conclusion The early symptoms of lung langershans cell histiocytosis are not typical,improve the pathological examination as early as possible to improve the efficiency of diagnosis and treatment.

关 键 词：朗格汉斯组织细胞增生症 诊断 治疗 文献复习 

分 类 号：R597[医药卫生—内科学]